| Product name: |
MCCB rabbit pAb |
| Reactivity: |
Human; Mouse;Rat |
| Alternative Names: |
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta) |
| Source: |
Rabbit |
| Dilutions: |
WB 1:500-2000 |
| Immunogen: |
Synthesized peptide derived from human MCCB AA range: 204-254 |
| Storage: |
-20C/1 year |
| Clonality: |
Polyclonal |
| Isotype: |
IgG |
| Concentration: |
1 mg/ml |
| Observed Band: |
60kD |
| GeneID: |
64087 |
| Human Swiss-Prot No: |
Q9HCC0 |
| Cellular localization: |
Mitochondrion matrix . |
| Background: |
This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008], |
